The Elephant In The Room
Karin S. Gilkison, MD, MPH, Barbara Burrall, MD
University of California, Davis Medical Center, Sacramento, CA
INTRODUCTION:
DISCUSSION
Elephantiasis nostras verrucosa is a dermatologic condition that is characterized by
hyperkeratotic, verrucous papules, plaques, and nodules with underlying woody fibrosis of
the dermis and subcutaneous tissue. It is a complication of chronic obstructive
lymphedema, which can slowly progress to produce massive enlargement of the affected
body parts, usually the lower extremities. We present a case of an older gentleman with a
classic presentation of chronic lower extremity lymphedema.
Elephantiasis nostras verrucosa is the sequela of chronic, non-filarial lymphedema, which is a
result of obstruction of lymphatic channels that carry fluids and waste materials from the
interstitial tissues to the venous circulation for removal and filtration. Secondary causes of
lymphedema include trauma, infection, radiation, surgery, lymph node dissection, lymph stasis,
and, as in this case, morbid obesity and chronic immobility. Our patient’s prior vein surgeries and
recurrent infections contributed as well. This condition can appear in any body region affected by
chronic lymphedema, but is typically manifested in the lower extremities. Elephantiasis nostras
verrucosa is often a clinical diagnosis confirmed by skin biopsy, which demonstrates a spectrum
of pseudoepitheliomatous hyperplasia, dermal fibrosis, dilated lymphatic vessels, and chronic
inflammation.
LEARNING OBJECTIVES:
1. Recognizing a classic presentation of Elephantiasis nostras verrucosa
3. Acknowledging the sequelae of untreated Elephantiasis nostras verrucosa, the
beneftis of early intervention, and methods used for treatment
Treatment remains a challenge, but centers around the reduction of the underlying lymphedema.
Early recognition of skin changes is critical to identify the need to treat. Leg elevation,
compression, and lymphedema pumping are beneficial, especially when utilized before chronic
skin changes manifest. Adjunctive therapies include oral antibiotic prophylaxis, oral and topical
retinoids, and topical keratolytics. Surgical modalities, including lymphaticovenular anasthamosis
and even amputation, may be considered.
CASE
The nodules and ulcers that may arise can be concerning for malignancy. In this case, the
nodule on the right lower leg was biopsied to rule out squamous cell carcinoma, including
verrucous carcinoma, and angiosarcoma.
2. Identifying clinical hallmarks of Elephantiasis nostras verrucosa
Presentation
A 70 year old chronically inactive, smoking, morbidly obese man with a history of bilateral
lower extremity vein stripping presented for examination of an exophytic skin nodule of
the right shin that had been increasing in size for the last three years. The patient
reported no malaise, fevers, weight loss, or night sweats. The lesion did not bleed or
ooze pus, and evolved on top of underlying chronic skin changes that had been present
for more than 40 years.
CLINICAL PEARLS
Identification and Treatment
Exam
Patient was morbidly obese. Other vital signs were unremarkable.
Cardiovascular and respiratory examinations were unremarkable.
Neurologic examination was unremarkable.
Skin and extremity examination revealed large, edematous legs with extensive, nontender, firm, minimally pitting, verrucous plaque that spared the ankle folds on his bilateral
lower extremities. On the right shin there was a large, 6x10cm fungating, cobblestone
nodule that was skin colored, woody, and non-fluctuant, but displayed significant erosion
with serous oozing.
This fungating skin lesion was biopsied for concern of squamous
cell carcinoma, including verrucous carcinoma, and angiosarcoma.
Chronic skin changes seen in elephantiasis start in areas with the
lowest lymph flow, such as the feet and legs in this sedentary patient
Laboratory Studies
A skin punch biopsy was performed. Pathology revealed edema and fibrosis throughout
the dermis. These was also an increased number of blood vessels oriented
perpendicular to the skin surface as well as patchy inflammation and hemosiderin laden
macrophages.

Elephantiasis nostra verrucosa is the sequela of chronic, non-filarial, obstruction of lymph
channels commonly caused by obesity, immobility, infection, trauma, prior vein stripping,
radiation, surgery, or lymph node dissection.

Clinical diagnosis can be made with history and skin exam, which typically reveals edematous
tissue and chronic development of non-tender, minimally pitting, firm or woody verrucous
plaques. Biopsy can confirm diagnosis, and rule out the development of secondary cancer.

Early recognition of skin changes is critical to identify the need to treat.

Primary treatment is centered around the reduction of lymphedema, including leg elevation,
compression, and lymphedema pumping.

Adjunctive therapies include oral antibiotic prophylaxis, oral and topical retinoids, and topical
keratolytics. Surgical treatment modalities include lymphaticovenular anasthamosis and even
amputation.
REFERENCES:
Clinical Course
Baird D, Bode D, Akers T, Deyoung Z. Elephantiasis Nostras Verrucosa (ENV): a complication of
congestive heart failure and obesity. J Am Board Fam Med. 2010 May-Jun;23(3):413-7.
Elephantiasis nostras verrucosa is typically a clinical diagnosis, but in this case was
confirmed with skin biopsy in order to rule out the development of cancer.
Dean SM, Zirwas MJ, Horst AV. Elephantiasis nostras verrucosa: an institutional analysis of 21 cases. J
Am Acad Dermatol. 2011 Jun;64(6):1104-10. Epub 2011 Mar 25.
The slowly progressive cutaneous changes of elephantiasis nostra verrucosa left
untreated may result in permanent disfigurement and gross dysfunction. With chronic
dermal inflammation and local collection of toxic metabolites, a small percentage may
progress into squamous cell carcinoma, including verrucous carcinoma, and
angiosarcoma.
Konia, Thomas, MD. Pathology slides of elephantiasis nostra verrucosa from this patient case included
as poster images. September 2011.
The patient in this case had failed home treatment with compression stockings and leg
elevation, and was referred to the lymphedema clinic for aggressive initiation of
lymphedema pumping. Surgical lymphaticovenular anasthamosis was not a treatment
option for this patient due to his history of prior vein stripping, and the benefits of
amputation did not outweigh the risks at this juncture. Topical retinoids and keratolytics
may be considered in the future.
Setyadi HG, Iacco MR, Shwayder TA, Ormsby A. Elephantiasis nostras verrucosa on the buttocks and
sacrum of two immobile men. Dermatol Online J. 2011 Feb 15;17(2):8.
Yoho RM, Budny AM, Pea AS. Elephantiasis nostras verrucosa. J Am Podiatr Med Assoc. 2006 SepOct;96(5):442-4.
Pathology slides at 100X, 200X and 300X. Routine hematoxylin and eosin stains show hyperkeratosis overlying diffuse dermal edema
with multiple dilated lymphatic vascular spaces, extravasated red blood cells, and scattered siderophages.
Zouboulis CC, Biczó S, Gollnick H, Reupke HJ, Rinck G, Szabó M, Fekete J, Orfanos CE. Elephantiasis
nostras verrucosa: beneficial effect of oral etretinate therapy. Br J Dermatol. 1992 Oct;127(4):411-6.